Diaz-Menindez Maximiliano, Chindris Ana-Maria, Mead-Harvey Carolyn, Li Yan, Butendieck Ronald R, Chirila Razvan M, Britt Katherine L, Berianu Florentina
Division of General Internal Medicine, Mayo Clinic, Scottsdale, AZ 85259, USA.
Division of Endocrinology, Mayo Clinic, Jacksonville, FL 32224, USA.
Diagnostics (Basel). 2025 Jun 21;15(13):1577. doi: 10.3390/diagnostics15131577.
: Autoimmune inner ear disease (AIED) causes sensorineural hearing loss that classically presents as fluctuating, asymmetric loss of hearing. Associated vestibular and other ear symptoms can be present in many patients. First-line treatment of AIED is high-dose corticosteroids. AIED can present either as a primary condition limited to ear involvement or secondary, as part of an underlying systemic autoimmune rheumatic disease, the most common of which include vasculitis and relapsing polychondritis. We described our cohort of primary AIED, including demographics, treatment, and outcomes. We excluded from this review sensorineural hearing loss in the context of vasculitis and relapsing polychondritis. : We performed a chart review of patients with the diagnosis of AIED at Mayo Clinic and compared the cohort by sex. : Thirty-one patients met the inclusion criteria. The mean age was 48.5 years, and 17 were men. Patients were initially evaluated at the Department of Otorhinolaryngology or Internal Medicine, and 29 patients were subsequently referred to the Department of Rheumatology, with a mean of 12.2 weeks after the first evaluation. Treatment with corticosteroids showed improvement in hearing and vestibular symptoms during the first month but no further improvement by the end of the third month. Other immunosuppressive medications were used with various degrees of response. Methotrexate was the second most used therapy, with 11 of 17 patients reporting an improvement in symptoms. : Corticosteroid therapy is an effective initial treatment for AIED and should be followed with corticosteroid-sparing agents to prevent further damage to the cochlea.
自身免疫性内耳疾病（AIED）可导致感音神经性听力损失，典型表现为波动性、不对称性听力丧失。许多患者还可能伴有前庭及其他耳部症状。AIED的一线治疗方法是大剂量使用皮质类固醇。AIED既可以是仅限于耳部受累的原发性疾病，也可以是继发性的，作为潜在的全身性自身免疫性风湿性疾病的一部分，其中最常见的包括血管炎和复发性多软骨炎。我们描述了我们的原发性AIED队列，包括人口统计学、治疗方法和治疗结果。我们在本综述中排除了血管炎和复发性多软骨炎背景下的感音神经性听力损失。我们对梅奥诊所诊断为AIED的患者进行了病历回顾，并按性别对队列进行了比较。31名患者符合纳入标准。平均年龄为48.5岁，其中17名男性。患者最初在耳鼻喉科或内科接受评估，随后有29名患者被转诊至风湿科，首次评估后平均12.2周。使用皮质类固醇治疗在第一个月内听力和前庭症状有所改善，但在第三个月末没有进一步改善。使用了其他免疫抑制药物，反应程度各不相同。甲氨蝶呤是第二常用的治疗药物，17名患者中有11名报告症状有所改善。皮质类固醇治疗是AIED有效的初始治疗方法，随后应使用可减少皮质类固醇用量的药物以防止对耳蜗造成进一步损害。
